Parkinson’s Australia have taken PSP and MSA under their wing and on the PSP web site there is several information sheets about MSA that can be downloaded or purchased
These are things not normally highlighted in traditional literature about
Multiple System Atrophy. This list was compiled from the collective
experience (since 1995) of hundreds of members of the shydrager online
support group for MSA founded by Vanderbilt University Autonomic Dysfunction
Center staff and currently hosted at http://groups.yahoo.com/group/shydrager
1. PREVENT INFECTIONS: MSA patients often register a body temperature that is a
degree or more below normal. Because of this a fever may not be detected. Any
sudden worsening of symptoms or confusion could be a sign of infection. An even
lower temperature than the normally (MSA) low temperature can also be a sign of
infection. Be vigilant about preventing and detecting all types of infections.
Urinary tract(UTI) and lung infections(PNEUMONIA) are very common. Stay well
hydrated (64 oz fluid per day) to prevent urinary tract infections. Stay dry
and shift weight often to prevent skin pressure sores. Swallow carefully, avoid
choking and aspiration. Puree foods when swallowing becomes difficult. Get
speech and swallowing therapy to avoid aspiration pneumonia.
2. WATCH FOR SLEEP AND BREATHING PROBLEMS: Sleep and breathing problems are
very common in MSA patients. Sleep apnea (momentary lapses in breathing),
respiratory stridor (noisy breathing), REM behavior disorder (shouting and
acting out dreams) and excessive daytime sleepiness can be among the earliest
symptoms of MSA. Sleep apnea is very serious and can lead to sudden death
during sleep. Ask your bed partner if you snore, breathe loudly in your sleep,
talk in your sleep or act out your dreams. Ask to be referred to a sleep
specialist for a sleep study, often this can be performed in your own home.
Not getting a good night’s sleep can make other MSA symptoms seem worse both
for the patient and the caregiver. Sleep and breathing problems can be easily
treated with a CPAP or Bi-Pap – this is a mask with pressurized air worn at
night.
3. BE AWARE OF SITUATIONS THAT CAN AFFECT BLOOD PRESSURE:
Blood pressure can DROP suddenly(hypotension): a. After getting up in the
morning – drink a large glass of water before getting out of bed to raise your
blood pressure. b. During a bowel movement – use a foot stool in front of the
toilet to keep blood pressure up. c. After a large meal – eat 5 or 6 small
meals instead of larger meals d. After standing in one place for a long time –
sit whenever possible.
Blood Pressure can SPIKE UPWARD suddenly(hypertension): a. When lying flat
(supine) during sleep – raise the head of the bed 4 to 6 inches higher than
the foot of the bed at night to prevent this. Further note on blood
pressure: If a patient’s blood pressure is to high laying down then simply
sitting them up can bring it back down.
4. BE CAREFUL OF SURGERY: If an MSA patient is to have any surgery, a local
anesthetic should be the preferred choice if possible. If general anesthesia is
necessary, ensure that the anesthesiologist knows that MSA affects the
autonomic nervous system. The MSA patient must be well hydrated via IV before
and during surgery to maintain a safe blood pressure. Another note on surgery:
Prostate surgery should only occur if the urologist and neurologist have had a
consultation with each other.
5. BE CAREFUL WITH OVER-THE-COUNTER COLD/FLU/ALLERGY MEDICATIONS: Many of these
medications contain pseudoephedrine that can affect the heart and raise blood
pressure, leading to stroke. They also may interact with prescribed
medications. Be very careful and consult your doctor before mixing any
medications, including over-the-counter remedies or even vitamins.
6. PREVENT FALLS: Preventing falls is very important, if you fall and break a
bone you may become bedridden and more prone to infections. When it becomes
appropriate, use a cane, walker or wheelchair. Install grab bars in the
bathroom, use a raised toilet, use a shower chair or bench, use a hoyer lift
for transfers.
7. AVOID EXTREME HEAT OR COLD: MSA can affect the body’s ability to sweat and
to maintain a proper core temperature. It’s important to stay cool during hot
or humid weather and stay warm during cooler weather. Also avoid very hot baths
and showers.
8. EXERCISE! It’s important to maintain strength and flexibility for as long as
possible. Do range of motion exercises and any gentle exercise that can be done
when sitting. Water exercises are easy to do and very helpful. Practise speech
exercises along with the other exercises to help maintain strength and clarity
of voice for as long as possible. Ask for physical and occupational and
speech therapy as these are all known to be beneficial to MSA patients.
9. PREPARE FOR EMERGENCY ROOM VISITS: Keep a short description of your medical
history, a description of MSA, and your most recent medications list handy so
you can take it with you to any new doctors’ appointments, hand it over in an
emergency room, give it to caregivers, etc. If you are in any of those
situations, it’s much easier to have something pre-prepared, especially if
you’re talking to people who have never heard of MSA.
10. BE READY TO ADVOCATE AND TO COORDINATE A TEAM OF DOCTORS: Be ready to
“advocate” with your doctors, or ask a trusted friend, family member or
caregiver to play this role. MSA is a complex disorder and not every doctor
will have heard of it, find a doctor you trust who is willing to learn. There
is literature available and there are known MSA expert neurologists who can
act as consultants. At each appointment try to focus on 1 or 2 concerns to get
them addressed adequately. Have a buddy with you who will make sure you’re
heard! Often your general practitioner can help play the coordinator role by
referring you to other experts such as a neurologist, internist, cardiologist,
urologist etc.
* Autonomic failure involving urinary incontinence (inability to
control the release of urine from the bladder, with erectile
dysfunction in males) or an orthostatic decrease of blood pressure
within 3 min of standing by at least 30 mm Hg systolic or 15
mm Hg diastolic and
* Poorly levodopa-responsive parkinsonism (bradykinesia with
rigidity, tremor, or postural instability) or
* A cerebellar syndrome (gait ataxia with cerebellar dysarthria, limb
ataxia, or cerebellar oculomotor dysfunction)”
* Parkinsonism (bradykinesia with rigidity, tremor, or postural
instability) or
* A cerebellar syndrome (gait ataxia with cerebellar dysarthria, limb
ataxia, or cerebellar oculomotor dysfunction) and
* At least one feature suggesting autonomic dysfunction (otherwise
unexplained urinary urgency, frequency or incomplete bladder
emptying, erectile dysfunction in males, or significant orthostatic
blood pressure decline that does not meet the level required in
probable MSA) and
* At least one of the additional features shown in table 3″
“Table 3 Additional features of possible MSA
Possible MSA-P or MSA-C
* Babinski sign with hyperreflexia
* Stridor
Possible MSA-P
* Rapidly progressive parkinsonism
* Poor response to levodopa
* Postural instability within 3 y of motor onset
* Gait ataxia, cerebellar dysarthria, limb ataxia, or cerebellar
oculomotor dysfunction
* Dysphagia within 5 y of motor onset
* Atrophy on MRI of putamen, middle cerebellar peduncle, pons, or
cerebellum
* Hypometabolism on FDG-PET in putamen, brainstem, or cerebellum
Possible MSA-C
* Parkinsonism (bradykinesia and rigidity)
* Atrophy on MRI of putamen, middle cerebellar peduncle, or pons
* Hypometabolism on FDG-PET in putamen
* Presynaptic nigrostriatal dopaminergic denervation on SPECT or PET
MSA = multiple system atrophy; MSA-P = MSA with predominant
parkinsonism;
MSA-C = MSA with predominant cerebellar ataxia; FDG = [18F]
fluorodeoxyglucose.”
Table 4 Features supporting (red flags) and not supporting a
diagnosis of MSA
Supporting features
* Orofacial dystonia
* Disproportionate antecollis
* Camptocormia (severe anterior flexion of the spine) and/or Pisa
syndrome (severe lateral flexion of the spine)
* Contractures of hands or feet
* Inspiratory sighs
* Severe dysphonia
* Severe dysarthria
* New or increased snoring
* Cold hands and feet
* Pathologic laughter or crying
* Jerky, myoclonic postural/action tremor
Nonsupporting features
* Classic pill-rolling rest tremor
* Clinically significant neuropathy
* Hallucinations not induced by drugs
* Onset after age 75 y
* Family history of ataxia or parkinsonism
* Dementia (on DSM-IV)
* White matter lesions suggesting multiple sclerosis
MSA = multiple system atrophy; DSM-IV = Diagnostic and Statistical
Manual of Mental
Disorders, Fourth Edition.”
What are the differences between this revised criteria (’08, second
consensus) and the previous criteria (’98, first consensus)? I don’t
know if anyone remembers but the ’98 criteria had fairly elaborate
tables of things called “features” and “criteria.” Fortunately
that’s been left behind in the revised criteria.
For probable MSA, the criteria are simplified. This
diagnosis “requires a reduction of systolic blood pressure by at
least 30 mm Hg or of diastolic blood pressure by at least 15 mm Hg
after 3 minutes of standing from a previous 3-minute interval in the
recumbent position. … (T)his is a more pronounced decrease of blood
pressure than recommended previously in the American Autonomic
Society (AAS)–AAN consensus statement on the definition of
orthostatic hypotension. … This is to ensure a high level of
accuracy in the diagnosis of MSA, because the disease is a grave one
and carries the prognosis of a markedly shortened life span.”
For possible MSA, the criteria are simplified and “has been changed
to require at least one feature suggesting autonomic dysfunction in
addition to parkinsonism or a cerebellar syndrome. … This change,
particularly the requirement of a feature suggesting autonomic
dysfunction, hopefully will decrease the false positives that
characterize clinical diagnosis in the early stages of the
disorder.” For possible MSA, “it is possible now to use both
clinical and imaging results to buttress the diagnosis in subjects
with parkinsonian features or cerebellar dysfunction plus autonomic
symptoms that do not meet the level needed for the diagnosis of
probable MSA.”
— In shydrager@yahoogroups.com, “rriddle_travel” <rriddle@…>
wrote:
We’ve been waiting for the consensus report ever since the conference
among MSA diagnosticians occurred last year. It’s finally out. Can
anyone obtain a free copy (of the PDF)?
Based upon the abstract below, we can see that there are still two
types of MSA — MSA-P (predominant parkinsonism) and MSA-C
(predominant cerebellar ataxia. And:
“Definite MSA requires neuropathologic demonstration of CNS alpha-
synuclein-positive glial cytoplasmic inclusions with
neurodegenerative changes in striatonigral or olivopontocerebellar
structures.”
“Probable MSA requires a sporadic, progressive adult-onset disorder
including rigorously defined autonomic failure and poorly levodopa-
responsive parkinsonism or cerebellar ataxia.”
“Possible MSA requires a sporadic, progressive adult-onset disease
including parkinsonism or cerebellar ataxia and at least one feature
suggesting autonomic dysfunction plus one other feature that may be a
clinical or a neuroimaging abnormality.”
Robin
Neurology. 2008 Aug 26;71(9):670-6.
Second consensus statement on the diagnosis of multiple system
atrophy.
Gilman S, Wenning GK, Low PA, Brooks DJ, Mathias CJ, Trojanowski JQ,
Wood NW, Colosimo C, Dürr A, Fowler CJ, Kaufmann H, Klockgether T,
Lees A, Poewe W, Quinn N, Revesz T, Robertson D, Sandroni P, Seppi K,
Vidailhet M.
Department of Neurology,
University ofMichigan,Ann Arbor, MI.BACKGROUND: A consensus conference on multiple system atrophy (MSA)
in 1998 established criteria for diagnosis that have been accepted
widely. Since then, clinical, laboratory, neuropathologic, and
imaging studies have advanced the field, requiring a fresh evaluation
of diagnostic criteria. We held a second consensus conference in 2007
and present the results here.
METHODS: Experts in the clinical, neuropathologic, and imaging
aspects of MSA were invited to participate in a 2-day consensus
conference. Participants were divided into five groups, consisting of
specialists in the parkinsonian, cerebellar, autonomic,
neuropathologic, and imaging aspects of the disorder. Each group
independently wrote diagnostic criteria for its area of expertise in
advance of the meeting. These criteria were discussed and reconciled
during the meeting using consensus methodology.
RESULTS: The new criteria retain the diagnostic categories of MSA
with predominant parkinsonism and MSA with predominant cerebellar
ataxia to designate the predominant motor features and also retain
the designations of definite, probable, and possible MSA. Definite
MSA requires neuropathologic demonstration of CNS alpha-synuclein-
positive glial cytoplasmic inclusions with neurodegenerative changes
in striatonigral or olivopontocerebellar structures. Probable MSA
requires a sporadic, progressive adult-onset disorder including
rigorously defined autonomic failure and poorly levodopa-responsive
parkinsonism or cerebellar ataxia. Possible MSA requires a sporadic,
progressive adult-onset disease including parkinsonism or cerebellar
ataxia and at least one feature suggesting autonomic dysfunction plus
one other feature that may be a clinical or a neuroimaging
abnormality.
CONCLUSIONS: These new criteria have simplified the previous
criteria, have incorporated current knowledge, and are expected to
enhance future assessments of the disease.
PMID: 18725592